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Details on Person Mitochondrial methylmalonate semialdehyde dehydrogenase (ALD...

Class:IdSummation:178951
_displayNameMitochondrial methylmalonate semialdehyde dehydrogenase (ALD...
_timestamp2010-02-12 15:25:52
created[InstanceEdit:179407] Gopinathrao, G, 2006-04-27 13:13:48
modified[InstanceEdit:508464] D'Eustachio, P, 2010-02-12
textMitochondrial methylmalonate semialdehyde dehydrogenase (ALDH6A1) catalyzes the reaction of methylmalonate semialdehyde, NAD+, and CoA to form propionyl-CoA, CO2, and NADH + H+. A human ALDH6A1 gene has been cloned. Its sequence is closely homologous to that of the better-characterized rat enzyme (Kedishvili et al. 1992) and a missense mutation in a normally well-conserved codon has been found in the allele of the gene from a patient with a defect in methylmalonic semialdehyde dehydrogenase activity (Chambliss et al. 2000).
(summation)[Reaction:70893] methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+ [Homo sapiens]
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